Sunday, December 18, 2011

In Memory of Sahib Singh Vaseer: Endocarditis and Bicuspid Aortic Valves

Sahib Singh Vaseer
July 7, 1995 - March 23, 2006
On the Bicuspid Aortic Foundation's website is the heartbreaking story of a little boy, Sahib Singh Vaseer, whose life was taken by infection just over five years ago. No one knew that Sahib was born with a bicuspid aortic valve, increasing his risk of infection attacking his heart.

In the words of his family, " Our  lives  were  shattered  and  we were devastated. The  earth had  moved  from  beneath  our  feet....  How  could   such  an active  boy  go  away  like  this?"  

At this time of holidays and family gatherings, we are painfully aware of those who are missing - taken from their loved ones by various complications of Thoracic Aortic Disease.  We need to be reminded that infection is a serious threat and remains a very real risk to those born with bicuspid aortic valves.

Following is a video clip about the risk of infection, taken from the Bicuspid Aortic Foundation's 2011 Conference. We share it here in loving memory of a Sahib.

Aortic Valve Stenosis, the Risk of Under-Treatment, and a "Boot-Shaped Heart"

Some hospitals have beautiful natural settings, and we recently found ourselves at one of them - high up on a hillside overlooking the sparkling, blue Pacific Ocean. We were there recently for an echo, and I remembered again the first time I came there, with my very sick husband. The beauty of that setting was rather wasted on us then.

Aortic Stenosis and Heart Failure
My husband had a life-long heart murmur, but never had the "classic" warning symptoms of valve disease - shortness of breath, fatigue, swollen ankles due to fluid retention. Somehow his body compensated so well, that no one knew there was just enough fluid gathering in his lungs to make an inviting place for bacteria to grow.  I was terrified as the fever spiked and his body was wracked with chills. It was off to the ER - abruptly he was in full blown double pneumonia and then obvious heart failure.

The pneumonia was very serious - I remember being told there was only one other drug choice if the antibiotic didn't work - it did. I understood how deadly pneumonia can be even in younger people then. Jim Henson, creator of the Muppets, died of pneumonia later that year.

We began a very personal crash course on aortic valve disease. I remember his Internist drawing a picture of a heart and showing us where all the valves were. We learned it was his aortic valve that had a problem - the valve that had made just a little murmur when he was born had gradually become more noisy and now made a horrendous rumbling, even to my untrained ear without a stethoscope.

We learned it was aortic stenosis - calcification had built up on the two leaflets he was born with until now the valve opening was only 0.5 cm2 - classified as critical.

Over the years, his heart muscle had been very strong and  pushed the blood out to his body through that bicuspid aortic valve as gradually it narrowed. Working so hard, his left ventricular muscle thickened and became misshapen - they told us his heart was shaped like a boot.

Aortic Stenosis Can Be Deadly
In the video below, from the Foundation's 2011 Conference, statistics are presented about aortic stenosis, and how quickly death follows once it becomes severe - half of those with untreated severe aortic stenosis die within 2 years of their diagnosis. Given how suddenly and severely a crisis developed in my husband, I know he did not have even that much time.

Under-Treatment of Aortic Stenosis
The magnitude of the aortic stenosis problem is shocking: 300,000 Americans that need treatment today, but only 80,000 surgeries done annually. Why? It is important for individuals and families to know that aortic stenosis can be under-treated. There is no medical treatment, and if timely evaluation and discussion of surgery does not happen, the patient may become too sick. Percutaneous valve insertion (TAVI) has just been approved in the US for those too sick to have surgery - but that approval does not include those with BAVs. This video clip from the Foundation's 2011 Conference highlights the concern about under treatment and the possibility for excellent outcomes even at older ages.

Be Well Informed and Avoid Under-Treatment
Aortic stenosis is a very serious condition - being well informed and finding expert care is important. Taking advantage of the information resources available today can make such a difference. It is possible for individuals to consult with surgeons directly, obtaining multiple opinions as needed to understand their options, in order to avoid under-treatment. At the same time, the aorta should be evaluated for enlargement in those who have BAV or come from a BAV family.

My husband's heart murmur was never really "followed" most of his life. He seemed well, and we just did not know any better. As I walked across from the parking lot into the hospital, I remembered how little we knew, how vulnerable we were those many years ago. He is among those fortunate ones whose hearts "remodel" and come out of failure. He fully recovered after his failed BAV was replaced. But that does not always happen, leaving otherwise healthy people impaired by a damaged heart. In order to receive the full benefit of aortic valve replacement or insertion, the heart itself should not have been injured by the effects of aortic stenosis.

May all those with aortic stenosis today receive the information and help they need to keep them safe, and have well-timed intervention to keep widely open that vital flow of blood from their heart.

Best wishes,
Arlys Velebir
Bicuspid Aortic Foundation